Interstitial type granuloma annulare associated with Sjögren’s syndrome

We describe a case of granuloma annulare (GA) associated with Sjögren’s syndrome (SS) in a 69‐year‐old woman. She complained of erythematous plaques on the left forearm and neck in addition to dry eyes and mouth. The laboratory and clinical findings also fulfilled the criteria for diagnosis of SS. Histopathological examination revealed the features of interstitial type GA. It is not rare that granulomatous diseases are associated with autoimmune diseases. This case indicated that granulomatous diseases and SS are closely related and that GA should be recognized as a cutaneous manifestation associated with autoimmune diseases, including SS.     

Scarring of lupus miliaris disseminatus faciei: treatment with a combination of trichloroacetic acid and carbon dioxide laser

We present a case of a 35‐year‐old man having a 12‐month history of multiple reddish‐brown papules on the chin, forehead, cheeks, and eyelids. Histopathologic findings revealed epithelioid cell granulomas with central necrosis consistent with a diagnosis of lupus miliaris disseminatus faciei. After 9 months of combined treatment with ethambutol, rifampin, and pyrazinamide, most lesions gradually resolved but remained as severe disfiguring scars. After 10 sessions of treatments with 100% trichloroacetic acid and CO₂ laser, the lupus miliaris disseminatus faciei scars have been much improved and the patient has never experienced a recurrence of disease during subsequent years of follow‐up.     

Acquired ichthyosis associated with an overlap syndrome of systemic sclerosis and systemic lupus erythematosus

Acquired ichthyosis is a condition accompanying many systemic illnesses such as lymphoma, sarcoidosis, dermatomyositis and systemic lupus erythematosus (SLE). Overlap syndromes are defined as clinical entities which satisfy each of the diagnostic criteria of two different connective tissue diseases concurrently or consecutively. The coexistence of SLE with systemic sclerosis has been very rarely reported. We describe a 33-year-old woman with an overlap syndrome consisting of systemic sclerosis and SLE who developed ichthyosis on her extremities.     

Palisading granuloma presenting in a patient with systemic lupus erythematosus

A patient with systemic lupus erythematosus who developed a distinctive nodular eruption demonstrating typical features of palisading granuloma is presented herein. A 60-year-old woman was admitted complaining of an elastic-hard, indurated nodule on the right dorsal aspect of the metacarpophalangeal joint of her third finger with a history of several years. She was successfully treated for her lupus nephritis with oral predonisolone. She had experienced erythema on her cheeks after sun exposure and polyarthralgia for a decade. Laboratory examination revealed positive results for antinuclear antibody and rheumatoid factor. Peripheral blood cell counts showed leukocytopenia and lymphocytopenia. Results of hand X-ray were unremarkable. Histological examination of the skin biopsy specimen from the nodule revealed a structure composed of a central area of fibrinoid necrosis, surrounded by a middle zone of palisading cells and an outer zone of chronic lymphocyte infiltrate. These findings led us to the histological diagnosis of palisading granuloma. She was diagnosed as having systemic lupus erythematosus complicated with a rheumatoid nodule. She is currently under treatment with cyclophosphamide (50 mg/day) without exaggeration of her systemic lupus erythematosus and nodule.     

Postmenopausal frontal fibrosing alopecia in a Japanese woman with Sjögren's syndrome

Postmenopausal frontal fibrosing alopecia (PFFA) is a rare alopecia that develops in the frontoparietal scalp of postmenopausal women. Etiology of PFFA is unknown. Most of cases of PFFA have been reported in European and North American countries. Herein, we report a Japanese case of PFFA associated with Sjögren's syndrome. A 66‐year‐old woman had had slowly progressive, band‐like, scarring alopecia on her frontoparietal scalp. Hair follicles on the margin showed follicular keratosis. Histologically, fibrosis and lymphocytic infiltration were mild. This case suggests that PFFA may show mild inflammatory reaction and mild fibrosis in Japanese women. The association with immunological disorders including Sjögren's syndrome should be studied further.     

Case of Sjögren–Larsson syndrome with a large deletion in the ALDH3A2 gene confirmed by single nucleotide polymorphism array analysis

Sjögren–Larsson syndrome (SLS) is a neurocutaneous disorder inherited in an autosomal recessive fashion. SLS patients are characterized by lipid metabolism error, primarily leading to cardinal signs of ichthyosis, spasticity and mental retardation. Additional signs include short stature, epilepsy, retinal abnormalities and photophobia. More than 90 mutations of the ALDH3A2 gene have been reported for SLS, and such variants can be successfully detected at a rate of 94% by direct DNA sequencing. We performed direct sequencing of ALDH3A2 gene from the index patient, however, no mutation could be detected. HumanCytoSNPs12 array analysis and subsequent targeted single nucleotide polymorphism analysis revealed a novel deletion mutation at chromosome 17p11.2. This 67‐Kb region includes the first five coding exons of ALDH3A2, and is flanked by rs2245639 and rs962801. To the best of our knowledge, this mutation is novel and our findings broaden the mutation spectrum of ALDH3A2 causing SLS phenotype.     

Association of the Acral Type of Pustular Psoriasis,Sjögren's Syndrome,Systemic Lupus Erythematosus,and Hashimoto's Thyroiditis

We describe a case of a 53-year-old Japanese female suffering from Sjögren's syndrome, systemic lupus erythematosus, and Hashimoto's thyroiditis who developed pustules, erythema, and erosions on her fingers and toes. The histological specimen showed psoriatic changes. Indirect immunofluorescent study using anti-human IL (interleukin)-8 antibody produced positive staining patterns in the lesional epidermis. These findings suggested the diagnosis of acral pustular psoriasis. Diaminodiphenylsulfone at 75 mg orally daily for 20 days and the application of 0.12% betamethasone valerate ointment led to gradual improvement.     

Development of systemic sclerosis in a patient with systemic lupus erythematosus and topoisomerase I antibody

We describe a patient with systemic lupus erythematosus (SLE) associated with topoisomerase I (topo I, Scl-70) antibody, a specific marker for systemic sclerosis (SSc). SSc patients who produce this antibody have severe cutaneous and visceral involvement, and eventually have a poor prognosis. It is rare to find this antibody in patients with other collagen diseases. Only four SLE patients have been reported in the English language literature who were topo I antibody-positive but had no clinical evidence of scleroderma. The serum of our patient with SLE had precipitating topo I antibody from the onset of his disease. Twelve years after the onset of SLE, he developed proximal scleroderma and pulmonary fibrosis. This case reconfirms the prognostic significance of topo I antibody as a predictive marker, and indicates that SLE patients with topo I antibody require careful follow-up for future development of scleroderma.     

The circulating lymphocyte profiles in patients with discoid lupus erythematosus and systemic lupus erythematosus suggest a pathogenetic relationship

BACKGROUND: Discoid lupus erythematosus (DLE) and systemic lupus erythematosus (SLE) are chronic inflammatory diseases of unknown aetiology; the relationship of DLE with SLE has been a subject of debate for many years. OBJECTIVES; To find evidence for systemic immune activation in DLE by analysis of the immunophenotypic profiles of circulating lymphocytes, and to compare these changes with those in patients with SLE. METHODS: The immunophenotypic profile of peripheral blood lymphocyte subsets from 23 DLE patients without clinical or laboratory evidence of systemic disease, 25 SLE patients and 38 healthy donors was characterized by two-colour immunofluorescence flow cytometry analysis. None of the patients was receiving corticosteroid or immunosuppressive treatment. RESULTS: Patients with DLE had increased numbers of circulating HLA-DR+ CD3+ T cells and HLA-DR+ CD4+ T cells, indicating systemic T-cell activation, and an expansion of CD5+ CD19+ B cells. Decreased numbers of T-cell subsets expressing the differentiation markers CD11b and CD16/56, and of CD16/56+ natural killer cells were also found. In SLE, the changes were similar but more pronounced. In addition, a profound CD4+ T-cell lymphopenia and an increase of HLA-DR+ CD8+ T cells were found only in SLE. CONCLUSIONS: Our data provide evidence for systemic activation of the cellular immune system in patients with purely cutaneous DLE. Similarities in the lymphocyte immunophenotypic profiles in patients with DLE compared with SLE suggest that there are common immunopathological processes in these two conditions.     

Successful treatment with bosentan for refractory digital ulcers in a patient with systemic lupus erythematosus

We report a 32-year-old woman with a 12-year history of systemic lupus erythematosus. Physical examination revealed indurated plaques with small ulcers on her extremities and trunk, which were histologically diagnosed as lupus erythematosus profundus. On her arms and knees, multiple small calcified nodules were noted in the dermis and subcutis. An elevated level of anti-phosphatidylserine/prothrombin antibodies was noted. She had been suffering from digital ulcers on the left fourth finger. Despite conservative therapies, new ulcers appeared on other fingers. After the administration of bosentan, no new lesion has developed. Bosentan, recognized as a choice of treatments for digital ulcers in systemic sclerosis, is worth trying in systemic lupus erythematosus patients with refractory digital ulcers.     

Bullous systemic lupus erythematosus in a 6‐year‐old boy

Bullous systemic lupus erythematosus (BSLE) is a rare subepidermal blistering disorder characterized by an acute vesiculobullous eruption in a subset of individuals with systemic lupus erythematosus. BSLE most commonly affects young women and only rarely affects children. Herein we report a rare case of BSLE in a 6‐year‐old boy.     

Anti‐signal recognition particle antibody‐positive polymyositis in a patient with Sjögren's syndrome showing various types of annular erythema: Positive correlation between the activities of annular erythema and myositis

Annular erythema with Sjögren's syndrome (AESS) is occasionally found, especially in Asian patients, which is classified into three types. We present a case of Sjögren's syndrome showing various types of AESS with anti‐signal recognition particle antibody‐positive polymyositis. We successfully treated the eruption and myositis with a low dose of prednisolone. Every onset of annular erythema coincided with elevation of serum creatine kinase levels, which suggests the correlation between the activities of annular erythema and polymyositis.     

Sweet's syndrome associated with systemic lupus erythematosus: A case report and review of the literature

A 35‐year‐old Japanese female patient with systemic lupus erythematosus (SLE) presented with fever, erythematous papules and nodules, and polyarthralgia. Skin biopsy of a nodule was compatible with Sweet's syndrome. The papules/nodules were well treated with an oral glucocorticoid. Thirty cases of Sweet's syndrome associated with lupus erythematosus (LE) have been reported in the published work. The mean age was 34.2 years. They showed a higher male ratio (male : female, 1:2) compared with patients with SLE (1:9) and Sweet's syndrome (1:3.7). Sweet's syndrome may occur as a manifestation of LE, and a moderate dose of an oral glucocorticoid will result in a good response.     

Late‐onset anaphylactic reactions following i.v. cyclophosphamide pulse in a patient with systemic sclerosis and systemic lupus erythematosus overlap syndrome

Late‐onset anaphylactic reaction is a rare adverse effect of i.v. cyclophosphamide pulse (IVCY), which is caused by cyclophosphamide metabolites. We herein report a case of scleroderma and lupus overlap syndrome who developed anaphylactic reactions 3 h to 4 days after IVCY. Long time lapses between IVCY and appearance of symptoms are compatible with type I hypersensitivity to cyclophosphamide metabolites, appearing as late‐onset anaphylactic reaction. Albeit unusual, this is an important anaphylactic reaction to be aware of following IVCY.     

Drug‐induced systemic lupus erythematosus in a child after 3 years of treatment with carbamazepine

Drug‐induced lupus erythematosus (DILE) is a less severe variant of systemic lupus erythematosus (SLE) that generally resolves within weeks or months after the withdrawal of the implicated drug. DILE is unusual during childhood, with the most frequent age of presentation being at 50–70 years of age. Among different drugs, most commonly procainamide and hydralazine have been implicated as a cause of DILE. However carbamazepine (CBZ) is considered a low‐risk drug and very few cases have been reported in children. We describe the case of CBZ‐induced SLE in a 9‐year‐old girl following 3 years of CBZ therapy. This case report shows that drug‐induced SLE is an important side‐effect to be considered, even after long‐term treatment with CBZ, and also during childhood.     

SLE患者HSP90基因表达及其与IL-6相关性的研究

目的:通过检测系统性红斑狼疮(SLE)患者外周血中热休克蛋白90(HSP90)基因mRNA及IL-6的水平,探讨SLE患者HSP90基因的表达及其与IL-6间存在的相关性。方法:分别使用原位杂交法、双抗体夹心ELISA法检测45例SLE患者外周血中HSP90mRNA和IL-6的表达,并进行直线相关性分析。取15份健康人血样进行正常对照。结果:SLE活动期、非活动期PBMC中HSP90mRNA表达的平均光度值分别为0.350±0.225,0.221±0.040,均显著高于正常对照组0.114±0.009(P<0.01,P<0.05)。SLE活动期HSP90mRNA表达显著高于SLE非活动期(P<0.05)。SLE活动期、非活动期血清IL-6水平分别为(16.82±1.79)pg/mL,(12.58±1.12)pg/mL,均显著高于正常对照组(10.13±2.95)pg/mL(P<0.01,P<0.05)。SLE活动期IL-6水平显著高于SLE非活动期(P<0.01)。IL-6的表达与HSP90mRNA的含量之间呈直线正相关关系(r=0.413,P<0.01)。结论:SLE患者体内存在IL-6的过度表达,可导致HSP90mRNA表达的增多,进而导致HSP90蛋白增高,高表达的HSP90可参与SLE的自身免疫反应,这可能在SLE的发病机制中起着一定的作用。     

Asymptomatic Primary Sjögren's Syndrome in a Patient with Penicillin Drug Eruption

A 20-year-old woman visited our clinic because of acral numbness and skin eruptions after administration of oral penicillin. Serological tests revealed an increase of immunoglobulins (G & M), positive rheumatoid factor, anti-nuclear factor, anti-SSA and -SSB antibodies. Ophthalmologic and otolaryngological studies were consistent with those of Sjögren's syndrome. However, a skin biopsy specimen failed to show any specific changes for Sjögren's syndrome and the diagnosis of penicillin drug eruption was considered to be more preferable. We briefly discussed the coexistence of a drug allergy and asymptomatic connective tissue disease.     

系统性红斑狼疮患者CD62P、CD63表达与SLEDAI相关性分析

目的:观察系统性红斑狼疮(SLE)患者外周血血小板膜蛋白CD62P、CD63的表达,并探讨CD62P、CD63与SLE疾病活动指数(SLEDAI)之间的相关性。方法:采用流式细胞术对10例SLE患者外周血CD62P、CD63进行检测分析,以20例正常人作为对照。同时对该10例SLE患者进行SLEDAI评分。结果:SLE患者CD62P、CD63表达较对照组显著增高(P<0.01),CD62P表达与SLEDAI之间具有显著相关性(r=0.840,P=0.002)。结论:CD62P、CD63在SLE患者外周血中表达显著增高,CD62P可作为评估SLE疾病活动程度指标。